Hostname: page-component-586b7cd67f-tf8b9 Total loading time: 0 Render date: 2024-11-26T18:53:30.287Z Has data issue: false hasContentIssue false
  • English
  • Français

Outcomes for patients with an aortopulmonary window, and the impact of associated cardiovascular lesions

Published online by Cambridge University Press:  21 January 2005

Rajesh Bagtharia ,
Kalyani R. Trivedi ,
Harold M. Burkhart ,
William G. Williams ,
Robert M. Freedom ,
Glen S. Van Arsdell  and
Brian W. McCrindle
Rajesh Bagtharia
Affiliation:
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Kalyani R. Trivedi
Affiliation:
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Harold M. Burkhart
Affiliation:
Division of Cardiovascular Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
William G. Williams
Affiliation:
Division of Cardiovascular Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Robert M. Freedom
Affiliation:
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Glen S. Van Arsdell
Affiliation:
Division of Cardiovascular Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Brian W. McCrindle
Affiliation:
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Get access Rights & Permissions [Opens in a new window]

Abstract

An aortopulmonary window is a communication between the ascending aorta and pulmonary trunk in the presence of two separate arterial valves, and is often complicated by other associated defects. We sought to determine management and related outcomes in patients with this malformation.

We identified those patients presenting between 1969 and 1999 from the databases held in our Departments of Cardiology, Pathology and Cardiovascular Surgery. We obtained data relating to issues concerning demography, clinical findings, imaging, management and outcome.

The median age at presentation for the 42 patients identified, of whom 23 were female, was 62 days, with a range from birth to 6 years. Associated cardiac defects were present in 34 patients, including interruption of the aortic arch in 6 patients. The correct diagnosis was initially missed in 13 patients. Of the patients, six died without surgical repair, and 1 patient was lost-to-follow-up. Repair was performed in 35 patients, subsequent to repair of other defects in 4, in association with repair of other defects in 17, of whom 3 died, and as an isolated procedure in 14 patients, one of the latter being treated by transcatheter closure. Overall, there were 9 deaths, all in patients with complex associated defects, except 1 patient with a missed aortopulmonary window after repair of aortic coarctation. Kaplan-Meier estimates of survival were 81% at 3 months until 11.5 years, and 69% up to 21 years. Only the presence of interrupted aortic arch was independently associated with increased time-related mortality, the hazard ratio being 5.87 (p = 0.009).

The outcome for an isolated lesion is excellent. Mortality occurs mainly before repair, mostly with complex associated lesions, particularly interruption of the aortic arch.

Keywords

Aortopulmonary fenestrationaorticopulmonary septal defectaortopulmonary fistulacongenital heart disease
Type
Original Article
Information
Cardiology in the Young , Volume 14 , Issue 5 , October 2004 , pp. 473 - 480
Copyright
© 2004 Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Jacobs JP, Quintessenza JA, Gaynor JW, Burke RP, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: aortopulmonary window. Ann Thorac Surg 2000; 69: S44–S49.Google Scholar
Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol 1987; 59: 443–447.Google Scholar
Kirby ML, Gale TF, Stewart DE. Neural crest cells contribute to normal aorticopulmonary septation. Science 1983; 220: 1059–1061.Google Scholar
Besson III WT, Kirby ML, Van Mierop LH, Teaubeaut II JR. Effects of the size of lesions of the cardiac neural crest at various embryonic ages on incidence and type of cardiac defects. Circulation 1986; 73: 360–364.Google Scholar
McElhinney DB, Reddy VM, Tworetzky W, Silverman NH, Hanley FL. Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age. Am J Cardiol 1998; 81: 195–201.Google Scholar
Tkebuchava T, von Segesser LK, Vogt PR, et al. Congenital aortopulmonary window: diagnosis, surgical technique and long-term results. Eur J Cardiothorac Surg 1997; 11: 293–297.Google Scholar
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; 68: 128–133.Google Scholar
Ho YS, Gerlis LM, Anderson C. The morphology of aortopulmonary windows with regard to their classification and morphogenesis. Cardiol Young 1994; 4: 146–155.Google Scholar
Hew CC, Bacha EA, Zurakowski D, del Nido Jr PJ, Jonas RA. Optimal surgical approach for repair of aortopulmonary window. Cardiol Young 2001; 11: 385–390.Google Scholar
Talner CN. Report of the New England Regional Infant Cardiac Program by Donald C. Fyler, MD. Pediatrics 1980; 65 (Suppl): 375–461. Pediatrics 1998; 102: 258–259.Google Scholar
Samanek M, Voriskova M. Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study. Pediatr Cardiol 1999; 20: 411–417.Google Scholar
Bertolini A, Dalmonte P, Bava GL, Moretti R, Cervo G, Marasini M. Aortopulmonary septal defects: a review of the literature and report of ten cases. J Cardiovasc Surg (Torino) 1994; 35: 207–213.Google Scholar
Blieden LC, Moller JH. Aorticopulmonary septal defect: an experience with 17 patients. Br Heart J 1974; 36: 630–635.Google Scholar
Tiraboschi R, Salomone G, Crupi G, et al. Aortopulmonary window in the first year of life: report on 11 surgical cases. Ann Thorac Surg 1988; 46: 438–441.Google Scholar
Doty DB, Richardson JV, Falkovsky GE, Gordonova MI, Burakovsky VI. Aortopulmonary septal defect: hemodynamics, angiography, and operation. Ann Thorac Surg 1981; 32: 244–250.Google Scholar
Pinto RJ. Profile of aortopulmonary window in India – a study of 20 cases. Cardiol Young 1994; 4: 142–145.Google Scholar
Prasad TR, Valiathan MS, Shyamakrishnan KG, Venkitachalam CG. Surgical management of aortopulmonary septal defect. Ann Thorac Surg 1989; 47: 877–879.Google Scholar
Braunlin E, Peoples WM, Freedom RM, Fyler DC, Goldblatt A, Edwards JE. Interruption of the aortic arch with aorticopulmonary septal defect: an anatomic review. Pediatr Cardiol 1982; 3: 329–335.Google Scholar
Boonstra PW, Talsma M, Ebels T. Interruption of the aortic arch, distal aortopulmonary window, arterial duct and aortic origin of the right pulmonary artery in a neonate: report of a case successfully repaired in a one-stage operation. Int J Cardiol 1992; 34: 108–110.Google Scholar
Redington AN, Rigby ML, Ho SY, Gunthard J, Anderson RH. Aortic atresia with aortopulmonary window and interruption of the aortic arch. Pediatr Cardiol 1991; 12: 49–51.Google Scholar
Balaji S, Burch M, Sullivan ID. Accuracy of cross-sectional echocardiography in diagnosis of aortopulmonary window. Am J Cardiol 1991; 67: 650–653.Google Scholar
Smallhorn JF, Anderson RH, Macartney FJ. Two dimensional echocardiographic assessment of communications between ascending aorta and pulmonary trunk or individual pulmonary arteries. Br Heart J 1982; 47: 563–572.Google Scholar
Horimi H, Hasegawa T, Shiraishi H, Endo H, Yanagisawa M. Detection of aortopulmonary window with ventricular septal defect by Doppler color flow imaging. Chest 1992; 101: 280–281.Google Scholar
Alva-Espinosa C, Jimenez-Arteaga S, Diaz-Diaz E, et al. Diagnosis of Berry syndrome in an infant by two-dimensional and color Doppler echocardiography. Pediatr Cardiol 1995; 16: 42–44.Google Scholar
Rice MJ, Seward JB, Hagler DJ, Mair DD, Tajik AJ. Visualization of aortopulmonary window by two-dimensional echocardiography. Mayo Clin Proc 1982; 57: 482–487.Google Scholar
Satomi G, Nakamura K, Imai Y, Takao A. Two-dimensional echocardiographic diagnosis of aortopulmonary window. Br Heart J 1980; 43: 351–356.Google Scholar
Gross RE. Surgical closure of an aortic septal defect. Circulation 1952; 5: 858–863.Google Scholar
Di Bella I, Gladstone DJ. Surgical management of aortopulmonary window. Ann Thorac Surg 1998; 65: 768–770.Google Scholar
Hammon Jr JW, Merrill WH, Prager RL, Graham Jr TP, Bender Jr HW. Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair. Ann Thorac Surg 1986; 42: 17–21.Google Scholar
Hata H, Shiono M, Sezai Y, Sumitomo N, Otsuka M, Harada K. One-stage repair of interrupted aortic arch and aortopulmonary window. Ann Thorac Surg 1998; 65: 829–831.Google Scholar
Kawata H, Kishimoto H, Ueno T, Nakajima T, Inamura N, Nakada T. Repair of aortopulmonary window in an infant with extremely low birth weight. Ann Thorac Surg 1996; 62: 1843–1845.Google Scholar
Kitagawa T, Katoh I, Taki H, et al. New operative method for distal aortopulmonary septal defect. Ann Thorac Surg 1991; 51: 680–682.Google Scholar
Matsuki O, Yagihara T, Yamamoto F, Nishigaki K, Uemura H, Kawashima Y. New surgical technique for total-defect aortopulmonary window. Ann Thorac Surg 1992; 54: 991–992.Google Scholar
Messmer BJ. Pulmonary artery flap for closure of aortopulmonary window. Ann Thorac Surg 1994; 57: 498–501.Google Scholar
Schmid FX, Hake U, Iversen S, Schranz D, Oelert H. Surgical closure of aorto-pulmonary window without cardiopulmonary bypass. Pediatr Cardiol 1989; 10: 166–169.Google Scholar
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; 35: 165–167.Google Scholar
Tulloh RM, Rigby ML. Transcatheter umbrella closure of aorto-pulmonary window. Heart 1997; 77: 479–480.Google Scholar
Naik GD, Chandra VS, Shenoy A, et al. Transcatheter closure of aortopulmonary window using Amplatzer device. Catheter Cardiovasc Interv 2003; 59: 402–405.Google Scholar
Atiq M, Rashid N, Kazmi KA, Qureshi SA. Closure of aortopulmonary window with amplatzer duct occluder device. Pediatr Cardiol 2003; 24: 298–299.Google Scholar
Erez E, Dagan O, Georghiou GP, Gelber O, Vidne BA, Birk E. Surgical management of aortopulmonary window and associated lesions. Ann Thorac Surg 2004; 77: 484–487.Google Scholar
Collinet P, Chatelet-Cheront C, Houze de l’Aulnoit D, Rey C. Prenatal diagnosis of an aortopulmonary window by fetal echocardiography. Fetal Diagn Ther 2002; 17: 302–307.Google Scholar
Valsangiacomo ER, Smallhorn JF. Images in cardiovascular medicine. Prenatal diagnosis of aortopulmonary window by fetal echocardiography. Circulation 2002; 105: E192.Google Scholar