aortopulmonary septal defect


Summary: A developmental abnormality in which the spiral (aortopulmonary) septum failed to completely divide the TRUNCUS ARTERIOSUS into ASCENDING AORTA and PULMONARY ARTERY. This abnormal communication between the two major vessels usually lies above their respective valves (AORTIC VALVE; PULMONARY VALVE).

Top Publications

  1. Croti U, Braile D, Kozak M, Diaz Suarez G. Case 6/2007--the uncommon association between the aortopulmonary window and the aortic coarctation. Rev Bras Cir Cardiovasc. 2007;22:365-6 pubmed
  2. Barnes M, Mitchell M, Tweddell J. Aortopulmonary window. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011;14:67-74 pubmed publisher
    ..Long-term follow-up is indicated to look for recurrent lesions such as the development of branch pulmonary artery stenosis and arch obstruction. ..
  3. Singh A, Mehmood F, Romp R, Nanda N, Mallavarapu R. Live/Real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. 2008;25:96-9 pubmed publisher
    ..We report the usefulness of three-dimensional transthoracic echocardiography in the assessment of AP window in two newborns studied by us. ..
  4. Kostolny M, Schreiber C, Hess J, Lange R. Successful primary correction of tetralogy of fallot with pulmonary atresia and aortopulmonary window in a 2,220-g neonate with a valved bovine jugular vein conduit. Herz. 2004;29:710-2 pubmed
    ..Minneapolis, MN, USA). At 15-month follow-up, the patient is in excellent clinical condition without medication. On echocardiography, the conduit showed a mean gradient of 11 mmHg with first-degree insufficiency. ..
  5. Lee E. MDCT and 3D evaluation of type 2 hypoplastic pulmonary artery sling associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis. J Thorac Imaging. 2007;22:346-50 pubmed
    ..Multidetector computed tomography combined with 3-dimensional evaluation was particularly helpful in making a correct diagnosis of the complicated anatomic anomalies found in this case. ..
  6. Atik E. Case 4/2007: a sixteen-month-old infant with patent ductus arteriosus. Arq Bras Cardiol. 2007;89:122-3, 135-6 pubmed
  7. 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;30:306-8 pubmed publisher
    ..An atrial septal defect with shunt flow left to right was also found. Surgical repair was performed 4 weeks after birth, the postsurgical period was uneventful and the newborn was discharged without complications 7 days after surgery. ..
  8. Respondek Liberska M, Gradecka M, Czichos E. Significant fetal pulmonary regurgitation as a possible prenatal sign of aorto-pulmonary window in the fetus with intrauterine growth retardation. A case report. Fetal Diagn Ther. 2004;19:72-4 pubmed
  9. Rohit M, Nandakumar S, Bahl A, Kubba S, Talwar K. Transcatheter closure of aortopulmonary window. Indian Heart J. 2005;57:161-3 pubmed
    ..We report the case of a 9-year-old girl with aortopulmonary window, in whom transcatheter closure was performed successfully using Amplatzer ductal occluder device. ..

More Information


  1. Maeda E, Akahane M, Kato N, Hayashi N, Koga H, Yamada H, et al. Assessment of major aortopulmonary collateral arteries with multidetector-row computed tomography. Radiat Med. 2006;24:378-83 pubmed
    ..MDCT studies may provide an efficient road map for safe and successful selective catheterization and may substitute for conventional angiography in patients with considerable risks. ..
  2. Senzaki H, Asano H, Masutani S, Matunaga T, Ishido H, Taketatu M, et al. Anomalous origin of the left coronary artery from the main pulmonary artery associated with Berry syndrome. J Thorac Cardiovasc Surg. 2003;126:1645-7 pubmed
  3. Mandegar M, Saidi B, Roshanali F. Aortiopulmonary window: detection with multislice computed tomography. Pediatr Cardiol. 2010;31:306-7 pubmed publisher
    ..Echocardiograpy showed a low ejection fraction and a connection between the ascending aorta and the pulmonary artery. Multislice computed tomography confirmed the diagnosis. He underwent successful repair of the connection. ..
  4. Ulrich U, Schrickel J, Dorn C, Richter O, Lewalter T, Luderitz B, et al. Mayer-von Rokitansky-Küster-Hauser syndrome in association with a hitherto undescribed variant of the Holt-Oram syndrome with an aorto-pulmonary window. Hum Reprod. 2004;19:1201-3 pubmed
    ..To the best of our knowledge, this is only the second report in the scientific literature on the concurrence of MRKHS and HOS, and the first published case of HOS with an aorto-pulmonary window as the cardiac malformation. ..
  5. Yorioka H, Kasamatsu A, Kanzaki H, Kawataki M, Yoo S. Prenatal diagnosis of fetal left pulmonary artery sling. Ultrasound Obstet Gynecol. 2011;37:245-6 pubmed publisher
    ..To our knowledge, prenatal diagnosis of LPA sling has not been reported so far. We report a case in which LPA sling was diagnosed during fetal ultrasound examination. ..
  6. Naik G, Chandra V, Shenoy A, Isaac B, Shetty G, Padmakumar P, et al. Transcatheter closure of aortopulmonary window using Amplatzer device. Catheter Cardiovasc Interv. 2003;59:402-5 pubmed
    ..Transcatheter device closure of APW should be considered when anatomy is favorable in terms of location and size of the defect with absence of associated anomalies. ..
  7. Noonan P, Desai T, Degiovanni J. Closure of an aortopulmonary window using the Amplatzer Duct Occluder II. Pediatr Cardiol. 2013;34:712-4 pubmed publisher
    ..The Amplatzer Duct Occluder II provides an additional device for aortopulmonary window closure, but anatomy and defect characteristics dictate the most appropriate device. ..
  8. McMahon C, Dibardino D, Undar A, Fraser C. Anomalous origin of left coronary artery from the right pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch. Ann Thorac Surg. 2002;74:919-21 pubmed
    ..This report highlights the importance of closely assessing the coronary ostia in patients undergoing complex aortopulmonary window repair. ..
  9. Orün U, Aydin H, Ocal B, Senocak F, Ozisik K, Kutsal A. Aortopulmonary window associated with anomalous right coronary artery: a rare combination. Anadolu Kardiyol Derg. 2007;7:E3-4 pubmed
  10. Chiu I, Wu S, Lee M. One-stage repair of interrupted aortic arch and aortopulmonary window with an autologous arterial flap. J Card Surg. 1999;14:306-9 pubmed
    ..of a transaortic approach permitted both accurate planning of the appropriate size of pulmonary trunk flap and also facilitated easy repair of the aortopulmonary septal defect. The growth potential of both great arteries was optimized.
  11. Patel C, Smith G, Lane J. Tetralogy of Fallot with an aortopulmonary septal defect: prenatal diagnosis. J Ultrasound Med. 2007;26:1115-7 pubmed
  12. Mishra A, Gandhi H, Sharma P, Patel H, Parmar D, Sheth M, et al. Transposition of great arteries with aortopulmonary window: our surgical experience. Ann Thorac Surg. 2014;97:196-201 pubmed publisher
    ..In our experience early diagnosis and an arterial switch operation have been crucial in getting a favorable outcome in planning of this complex congenital heart disease. ..
  13. Bialkowski J, Akdeniz C, Celebi A. Transcatheter closure in two rare cases of left-to-right shunt with Cardio-O-Fix occluders. Cardiol Young. 2012;22:96-9 pubmed publisher
    ..The procedures were successful in both patients, and we achieved complete closure of the unwanted shunts. ..
  14. Mainwaring R, Reddy V, Perry S, Peng L, Hanley F. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals. Ann Thorac Surg. 2012;94:842-8 pubmed publisher
    ..We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed. ..
  15. Collinet P, Chatelet Cheront C, Houzé de l Aulnoit D, Rey C. Prenatal diagnosis of an aorto-pulmonary window by fetal echocardiography. Fetal Diagn Ther. 2002;17:302-7 pubmed
    ..The purpose of this report is to demonstrate the feasibility of antenatal diagnosis of this fetal malformation and help professionals who would be faced with such an unexpected prenatal image. ..
  16. Wong M, Chan L, Sim K. Interrupted aortic arch and aortopulmonary window demonstrated on 64-slice multidetector computed tomography angiography. Heart. 2007;93:95 pubmed
  17. Pascual Calleja I, Garcia D, Salazar J. [Aortopulmonary window]. An Pediatr (Barc). 2008;68:637 pubmed
  18. Das B, Pauliks L, Chan K. Anatomically corrected malposition of the great arteries in the setting of aortopulmonary window associated with holoprosencephaly. Pediatr Cardiol. 2006;27:175-176 pubmed publisher
  19. Gangana C, Malheiros A, Alves E, de Azevedo M, Bernardes R, Simões L. Aortopulmonary window--impact of associated lesions on surgical results. Arq Bras Cardiol. 2007;88:402-7 pubmed
    ..The presence of associated complex congenital heart disease is a bad prognostic factor in our series. ..
  20. Bradley S. Management of aortopulmonary collateral arteries in Fontan patients: routine occlusion is not warranted. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2002;5:55-67 pubmed
    ..Preoperative coil occlusion may decrease overall risk in such patients. Postoperative APC occlusion is a reasonable option in the patient with prolonged effusions after a Fontan operation, with no other correctable anatomic defects. ..
  21. Guglielmini C, Pietra M, Cipone M. Aorticopulmonary septal defect in a German shepherd dog. J Am Anim Hosp Assoc. 2001;37:433-7 pubmed
    ..Doppler echocardiography showed continuous and turbulent flow in the ascending aorta between the aorta and the pulmonary artery. Necropsy demonstrated an aorticopulmonary septal defect. ..
  22. Bhan A, Gupta M, Abraham S, Sharma R, Kothari S, Juneja R. Surgical experience of aortopulmonary window repair in infants. Interact Cardiovasc Thorac Surg. 2007;6:200-3 pubmed
    ..In our view, simple ligation without CPB should be avoided due to the possibility of residual APW and distortion of pulmonary artery. ..
  23. Chen F, Ge S. Aortopulmonary window demonstrated by real-time 3-dimensional echocardiography and color Doppler. J Am Soc Echocardiogr. 2007;20:1314.e7-8 pubmed
  24. Sinzobahamvya N, Asfour B, Boscheinen M, Photiadis J, Fink C, Schindler E, et al. Compared fate of small-diameter Contegras and homografts in the pulmonary position. Eur J Cardiothorac Surg. 2007;32:209-14 pubmed
    ..As Contegras is 'off-the-shelf' available, it constitutes a sound alternative to homograft for right ventricular outflow tract reconstruction in neonates and infants. ..
  25. Tzifa A, Papagiannis J, Qureshi S. Iatrogenic aortopulmonary window after balloon dilation of left pulmonary artery stenosis following arterial switch operation. J Invasive Cardiol. 2013;25:E188-90 pubmed
    ..The diagnostic process with cardiac magnetic resonance imaging and cardiac catheterization of such an unusual entity as well as the transcatheter management are discussed in detail. ..
  26. Asano M, Ukai T, Nomura N, Mishima A. Anatomical repair of aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery. J Card Surg. 2013;28:767-9 pubmed publisher
    ..Complete anatomical correction comprising division of the great arteries and transferring the RCA as an autologous flap to the aortic defect was successfully performed. ..
  27. Shamim A, Khan M, Atiq M, Khan M, Amanullah M. Surgical correction of aorto-pulmonary window: a rare and lethal cause of pulmonary hypertension. J Pak Med Assoc. 2011;61:836-8 pubmed
    ..Hospital stay was complicated because of pulmonary arterial disease making it an important reason for correction in the first few months of life. ..
  28. Malec E, Brzegowy P, Mroczek T. Surgical treatment of aortopulmonary window with tetralogy of Fallot. Scand Cardiovasc J. 2001;35:159-60 pubmed
    ..We report a case of the combined anomalies in which, possibly for the first time, the diagnosis was made by echocardiography alone, and in which successful surgical treatment was performed in early infancy. ..
  29. Bergstrom C, Saunders R, Hutchinson A, Lambert S. Iris hypoplasia and aorticopulmonary septal defect: a neurocristopathy. J AAPOS. 2005;9:264-7 pubmed
    ..It is important that opthalmologists be aware of the association between these two disorders since an aorticopulmonary septal defect is a life-threatening disorder. ..
  30. Hew C, Bacha E, Zurakowski D, del Nido P, Jonas R. Optimal surgical approach for repair of aortopulmonary window. Cardiol Young. 2001;11:385-90 pubmed
    ..01). Repair of aortopulmonary window can be done with excellent results. A pulmonary arteriotomy should be avoided. ..
  31. Srivastava A, Radha A. Transcatheter closure of a large aortopulmonary window with severe pulmonary arterial hypertension beyond infancy. J Invasive Cardiol. 2012;24:E24-6 pubmed
    ..without complications in short-term follow-up. ..
  32. Prem Sekar R, Bhima Shankar P, Cherian K. Transcatheter closure of a residual aortopulmonary window through internal jugular vein access. Indian Heart J. 2012;64:521-3 pubmed publisher
    ..Technical difficulty anticipated was unfounded although it was more cumbersome than femoral access. Left to right cardiac defects including PDA and AP window are amenable to transcatheter closure through internal jugular vein access. ..
  33. Trehan V, Nigam A. Percutaneous device closure of nonrestrictive aortopulmonary window in a 4 months' infant. Indian Heart J. 2008;60:254-6 pubmed
    ..We report percutaneous device closure of nonrestrictive AP window in a 4 months old, 4 kg child. To the best of our knowledge this is the youngest child to have undergone percutaneous device closure of an AP window. ..
  34. Miyaji K, Nagata N, Matsui H, Miyamoto T, Kitahori K. Successful Fontan procedure for asplenia with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2003;126:1648-50 pubmed
  35. Anselmi A, Langanay T, Laurent M, Leguerrier A, Flecher E. Transpulmonary correction of an isolated aortopulmonary window in an adult. J Card Surg. 2013;28:663-5 pubmed publisher
    ..Patch closure through a combined transpulmonary approach was therefore required. This case illustrates unique features of a late-presenting aortopulmonary window. ..
  36. Sasmazel A, Alkan T, Ersoy C, Paker T, Akcevin A, Bayer V, et al. [Our surgical experiences with aortopulmonary window]. Anadolu Kardiyol Derg. 2006;6:77-8 pubmed
  37. Trehan V, Nigam A, Tyagi S. Percutaneous closure of nonrestrictive aortopulmonary window in three infants. Catheter Cardiovasc Interv. 2008;71:405-11 pubmed publisher
    ..Percutaneous closure of AP window in each of the three patients was done by a different type of device i.e. duct occluder, muscular VSD occluder and perimembranous VSD occluder. ..
  38. Nukić S, Kusljugic Z, Masic I. [Aortic stenosis and atrial septal defect in pulmonary hypertension caused by aorto-pulmonary fenestration]. Med Arh. 2006;60:202-4 pubmed
    ..After repeated catheterization when patient was 23 years old the final diagnosis was established: Fenestra aortico pulmonalis, VSD, ASD, aortic stenosis gr II and pulmonary hypertension. The patient has been treating conservatively ..
  39. Chen M, Wu S. Images in cardiovascular medicine. Unclassified type of aortopulmonary window. Circulation. 2006;113:e703-4 pubmed
  40. Chang Y, Sung S, Kim H, Lee H. Anterior translocation of the right pulmonary artery for relief of airway compression in the repair of distal aortopulmonary window and interrupted aortic arch. Ann Thorac Surg. 2012;93:e159-61 pubmed publisher
    ..After 37 months of follow-up, no problem was encountered with her airway or RPA. We describe the technique used and the results obtained. ..
  41. Tissot C, da Cruz E, Beghetti M, Aggoun Y. Successful use of a new Amplatzer Vascular plug for percutaneous closure of a large aortopulmonary collateral artery in a pulmonary atresia with ventricular septal defect prior to complete repair. Int J Cardiol. 2007;116:e39-41 pubmed
    ..This new device is safe and effective for the occlusion of aortopulmonary collaterals, specifically if of large dimensions. ..
  42. Shah S, Sukhramwala R, Maheshwari S. Aortic atresia with aortopulmonary window simulating common arterial trunk. Cardiol Young. 2009;19:304-6 pubmed publisher
    ..This unusual association changes the anticipated physiology, and helps in the survival. We emphasize the fact that this combination simulates common arterial trunk, both clinically and on echocardiography. ..
  43. Kiran V, Singh M, Shah S, John C, Maheshwari S. Lessons learned from a series of patients with missed aortopulmonary windows. Cardiol Young. 2008;18:480-4 pubmed publisher
    ..A high index of suspicion for this lesion is warranted in cases of unexplained severe pulmonary arterial hypertension, and/or unexplained cardiac dilation. ..
  44. Apitz C, Kaulitz R, Sieverding L, Girisch M, Salehi Gilani S, Ziemer G, et al. [Echocardiographic diagnosis of the aorto-pulmonary window]. Ultraschall Med. 2007;28:189-94 pubmed
    ..Cardiac catheterisation is required only in cases with inadequate information about coronary artery anatomy or in older patients with pulmonary hypertension, to determine the patient's suitability for surgical correction. ..
  45. Ishibashi N, Shin oka T, Ishiyama M, Sakamoto T, Kurosawa H. Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Eur J Cardiothorac Surg. 2007;32:202-8 pubmed
    ..05). Hypoplastic central PA was a significant risk factor in this disease. The overall survival was improved by our current management strategy. Improved RV pressure after definitive repair appears to affect the long-term outcome. ..
  46. Tunks R, Steed R, Lodge A. Surgical approach to a rare case of double-outlet right ventricle and aortopulmonary window. Cardiol Young. 2016;26:172-4 pubmed publisher
    ..We present an interesting case of an infant prenatally diagnosed with this rare CHD. To our knowledge, this is the first report of complete repair in the neonatal period for such a combination of defects. ..
  47. Chen H, Tseng H, Huang J, Dai Z. Coexistence of left pulmonary artery sling and aortopulmonary window complicated with difficult airway-a rare congenital cardiopulmonary defect. Eur J Cardiothorac Surg. 2005;28:900-2 pubmed
    ..A case of two such rare lesions coexisting was not to be found in the literature review, so we report this case because of its rarity and clinical interest. ..
  48. Kervancioglu S, Soydinc S, Davutoglu V, Kervancioglu R, Sirikci A, Bayram M. Aortopulmonary window: a rare adult case demonstration by echocardiography, MRI, CE-MRA and angiography. Cardiovasc Intervent Radiol. 2004;27:175-8 pubmed
  49. Murin P, Sinzobahamvya N, Blaschczok H, 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;60:215-20 pubmed publisher
    ..8% - 27.6%). Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation. ..
  50. Franzen O, Le T, Cesnjevar R, Begemann P, Baldus S. Successful treatment of an adult patient with an aortopulmonary window and severe unilateral pulmonary hypertension. Congenit Heart Dis. 2009;4:478-80 pubmed publisher
    ..Even though the vasculature of the left lung was severely damaged preoperatively, the resulting pressure in the lung after surgical correction was only mildly elevated. ..
  51. Kornyei L, Kiraly L, Szatmari A. [Berry syndrome: successful neonatal repair of a rare complex cardiac malformation]. Orv Hetil. 2004;145:963-6 pubmed
    b>Aortopulmonary septal defect, interrupted aortic arch, aortic origin of the right pulmonary artery, intact ventricular septum, and patent ductus arteriosus (Berry Syndrome) is a rarely reported malformation...
  52. Kanter K, Vincent R. Management of aortopulmonary collateral arteries in Fontan patients: occlusion improves clinical outcome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2002;5:48-54 pubmed
  53. Okamura T, Nagashima M, Shikata F. Primary repair of aortopulmonary window with an interrupted aortic arch in a very low-birth-weight premature neonate. Pediatr Cardiol. 2011;32:221-3 pubmed publisher
    ..We describe the diagnosis, surgery, and postoperative course of this rare occurrence. ..